Showing posts with label
Pelizaeus-Merzbacher disease (PMD).
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Showing posts with label
Pelizaeus-Merzbacher disease (PMD).
Show all posts
By Marcy White
I hate Pelizaeus-Merzbacher disease (PMD).
On March 21, 2003, I was told that PMD was the cause of my son Jacob’s many medical problems. I’ve hated the disease ever since. When I learned that it is degenerative and would continue to burrow into my son’s brain and nervous system like a voracious mole, until it ate away his abilities to think, to breathe and to live, I detested PMD. And when I witnessed my son on a ventilator two months ago because the disease had so weakened his immune system that a cold required him to have mechanical assistance to stay alive, my hate relationship was in full swing.
These days, I can’t think of PMD without my heart twisting with loathing. This is hate to the extreme. When Jake was hospitalized for the first three months of his life, I repeatedly said it wasn’t fair. Why was he, an innocent newborn with a whole life of possibilities ahead of him, suffering such excruciating pain and struggling so hard to breathe? It didn’t take long for me to realize that the saying “life isn’t fair”—often used half-heartedly by my parents when I complained as a teen about not being allowed to go to a party with friends—is 100 per cent accurate. Life isn’t fair and my son’s disease is proof of it.
Life’s cruelty and my abhorrence of PMD crashed into our house when I had to explain to my twin daughters that Jake’s PMD was worsening.
The fact that Jacob has PMD is wrong. The rapid change in his health is completely undeserved. And the fact that Jamie and Sierra have to experience all this, alongside their brother, is unreasonable in its heartlessness. At ten years old, Jamie and Sierra are forced to witness this injustice every day.
When Sierra and Jamie were six years old, they gave their friends an impromptu genetics lesson by explaining the inheritance pattern of the disease that affects their big brother. They’ve always known that Jacob’s care is difficult and there are nurses in his room every night to ensure his medical safety. But until recently, they didn’t realize that PMD is a fatal disease and that despite our efforts at researching a cure, their brother might not live long enough to benefit from medical breakthroughs.
I’ve learned that people adapt to changing conditions. I remember saying that I wouldn’t be able to stick a tube down my son’s throat and suction out the mucous that was blocking his airway and causing him to turn blue. Now, I do this every day and my hands remain surprisingly steady during the process. When Jamie used to hear the rumble of the suction machine, she would stop what she was doing and put her hands over her ears to block out the awful sound. But, last week, as I was threading the tube down Jake’s airway, Jamie held her brother’s hand and talked him through the entire procedure, even wiping away the tears that pooled at the edges of his eyes. There was so much injustice in that scene, but also a ton of love.
My daughters experience things on a daily basis that youngsters should not have to bear, and that is deplorably unfair. They are aware of the frailty of their brother’s lungs, they know how to read his oxygen monitors and understand what numbers indicate a dangerously high heart rate. They dissolve into tears when he needs to go to the emergency room because, in their experience, that is the start of a long hospitalization and a complete disruption of their lives.
Caring for Jacob is extremely challenging and scary at times. He can go from breathing quietly to sounding like he is drowning in his saliva in seconds. The level of oxygen in his blood can plummet from a safe 98 per cent to a dangerously low 77 per cent in the blink of an eye. The doctors and nurse practitioners have taught me how to care for him and when to intervene. So for the most part, I know how to “manage” my son’s precarious medical needs. I don’t like it, but I am adjusting to the new level of care that Jacob requires.
Late at night, when the house is quiet, save for the humming of the oxygen concentrator in Jake’s room, what keeps me awake is thinking about how PMD is affecting my twin daughters.
Prior to their births, I read a lot about siblings of kids with special needs. Everything I came across consistently stated that kids raised in homes with atypical children grew into empathic, sensitive and caring adults. I do believe that, and have already witnessed several examples of my daughters’ concern for the feelings of their friends. They display a level of compassion beyond their years.
Sierra and Jamie are forced to cope with things many adults are unable to fathom. My heart shatters when they come home from school crying because their friends don’t understand what it’s like to live with a disabled brother whose health is so fragile. I have to explain that most of their friends aren’t emotionally equipped to support them as they express their fears. And that, too, isn’t fair.
How do I hold it together when they ask questions like “Why doesn’t home feel like home anymore?” or “Why does my life have to be harder than everyone else’s at school?” or “Will a cure for PMD be discovered in time for Jake?”
These are questions about which little kids shouldn’t have to wonder, and issues from which I desperately wish I could shield my daughters. But I can’t. So we talk. In the car, on our way to an activity, we talk about why we don’t travel like their friends’ families. And at dinner, we talk about whether the auditorium where the girls are performing in a play is wheelchair accessible so Jacob can come and watch. And late at night, in the darkness of their rooms, we chat about how unfair it is that their brother has PMD, that he can’t tell us exactly what he’s thinking or feeling and that they are scared to be alone with him in case he stops breathing.
We love Jacob with every fibre of our beings. But we hate PMD.
This piece originally appeared in Her Magazine. You can follow Marcy White on her blog Cure PMD or read her book The Boy Who Can.
The Boy Who Can is a story about my friend Marcy White’s perseverance, creativity and courage in the face of her son Jacob’s life-threatening genetic disorder. The book begins with this “Letter to my Daughters,” Marcy’s twin daughters Sierra and Jamie (all above) who are not affected. It describes the natural hurt siblings can feel when so much of their parent’s time is taken up attending to the child with disability. An important message for all of us. Thanks Marcy!
Dear Sierra and Jamie:
You are too young for me to share this with you now but one day, when you are older, I will tell you how my heart hurt when you asked me why I loved your brother more than you. Your questions caught me off-guard and I’m not sure I expressed myself properly. I desperately need you to know that it is not true.
Being a parent is a constant juggling act, there are always so many balls in the air at risk of falling down and crashing to the floor. Parenting in a household with a “differently-abled” child like your brother is even more of a challenge, simply because his needs are different.
Jacob can’t speak so he doesn’t ask me to play with him. You do. Sometimes I feel guilty for spending more time with you, my mobile and verbal twins, than I do with your brother, who is dependent on others for absolutely everything.
I struggle with balancing my time between all my children. I am constantly asking myself how I can meet your needs without feeling that Jacob is constantly left out. At eight years old, you know so much about disease, more than any child your age should have to comprehend. You are aware that Pelizaeus-Merzbacher disease (PMD) is carried on the X chromosome and that you have two healthy Xs but Jake has a sick X that causes his PMD.
You understand how PMD affects your brother, how he can’t ask me a question, sit by himself or walk. You know that he can’t eat like you do, and needs to be fed with the tube that was surgically implanted into his stomach when he was a few weeks old.
Everything is so hard for him, while you girls can ride your bikes, dance and eat the things you help me bake.
Although it may seem as if Jacob and I spend a lot of time together, relatively little of it is “fun” time, and in fact, we have little time alone during the week. We have our special allotment on Saturdays, but as you know, that it never starts off as a fun morning.
As Emily’s overnight nursing shift nears its end, I try to make sure your breakfast is prepared and a supply of bibs is packed in a bag for Jacob, all his things organized and easily accessible as soon as the front door closes behind her. I run through my mental list of things that need to be done before the craziness begins in earnest.
From the moment Emily walks out of our house until your big brother is fastened in his car seat, you listen to Jacob crying and screaming. Our neighbors must wonder what awful torture I am inflicting on him. And I see you sitting there with your hands over your ears, trying to block out the noise. When you realize that won’t work, you begin to dance around in front of your brother in an eager attempt to console him and elicit a smile. You probably don’t realize this, but I stop what I’m doing and watch you. My heart swells with pride when I see you trying to help Jacob but I also feel like crying—I wish you didn’t have to watch your brother struggle so hard to make himself understood.
You should be able to enjoy a lazy weekend morning at home in your pajamas, cuddling in bed with your parents, not watching us run up and down the stairs, organizing your older sibling’s medical paraphernalia. Unfortunately—for all of us, but especially for Jacob—this is not what our life is like.
In order for me to hang out with Jacob, we have to leave our house. He screams incessantly when I try to play with him at home while you are present. As soon as we leave, he calms down. I have his full attention and together we can enjoy the outing. Maybe Jake knows that I can’t tune everyone else out at home and that he is forced to share my focus with you, the cats, and all the other millions of things that distract me. Regardless of the reason, when we are out, alone, we have fun. It’s “Mommy and Jakey” time.
And we have our weekly swimming. You know how your big brother loves the pool and has learned to swim somewhat independently, surprising the lifeguards and making us all so proud of him. If I’m nearby and not in the water with him, Jake complains and is unable to enjoy the activity; however, if I am in the water, reminding him to move his arms and kick his legs and encouraging his effort, he beams. He giggles and I feel his arms tighten around my neck in a delicious and intentional hug.
After our 45-minute swim, as we are getting dressed and ready to leave, my back aches. Holding him, walking with him on the ramp into and out of the pool, is taxing on a body that has endured more lifting and twisting than it can handle. But watching Jake swim the length of the pool, seeing his little legs break the surface during a kick and listening to his squeal of delight when his hand touches the wall at the end of a lap, is worth every pain.
After those outings, I return feeling like I have spent some quality time with my first born, the child I often believe does not get enough of my undivided attention.
It is so much easier for me to play with you, my beautiful darlings. You express your thoughts and questions so eloquently, and you don’t need me to help you move your leg if it slides off the footrest of your chair. I am in awe of your intelligence and imagination. I love watching you play elaborate games with your dolls as you create different experiences for them. I can sit and listen to your stories for hours. I marvel at the various masterpieces you create for me, each one an incredible work of art.
I drive you to and from your school while Jacob is bussed to and from his. I bathe you and help put on your pajamas. I tuck you into bed at night and comfort you when you wake with a nightmare. A caregiver bathes your brother because he is too heavy for me to carry. I am terrified that I might drop his wet and slippery body during the short transfer from his special shower chair to his changing table.
The same caregiver puts Jacob to sleep while I’m with you, listening to stories about your day. I crawl into bed with Jake for a few minutes of cuddling before he falls asleep. And it’s the nurse in his room who administers his medication and helps ease him back to sleep in the middle of the night when he wakes up with a fever.
But, when I put you into bed this evening, your question sliced through me like a jagged edge of broken glass: Why do you love Jacob more than us?
The answer, quite simply, is that I don’t love him more than you. And I don’t love you more than him. I love all of you the same. I just have to show it differently.
All my love,
Mommy
Marcy is speaking about her book at a Toronto event run by Three to Be's Parent Advocacy Link on May 28 from 7 to 9 p.m. at 452 Wilson Ave. RSVP to brenda@threetobe.org
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